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Glycoproteinoses, Pompe disease, and sialic acid storage diseases are characterized by a massive accumulation of unprocessed oligosaccharides and/or glycoconjugates in urine. The identification of these glycocompounds is essential for a proper diagnosis. In this study, we investigated the potential of MALDI‐TOF‐MS to identify glycocompounds present in urine from patients with different inborn errors of glycan metabolism. Urinary glycocompounds were permethylated, and analyzed using GC‐MS and MALDI‐TOF‐MS. In order to confirm tentative assignments, a second aliquot of urine was purified on a C18 Sep‐Pak cartridge and glycocompounds were desalted on a column of nonporous graphitized carbon. The glycocompounds were then sequentially on‐plate digested using an array of exoglycosidases. A range of disease‐specific oligosaccharides as well as glycopeptides was identified for all oligosacchariduria models. In addition, free sialic acid accumulated in urine from a patient suffering from French‐type sialuria, has been detected by a GC‐MS approach, which could be applied to other sialic acid storage diseases. This procedure is simple, and can be performed in few simple steps in less than 24 h. This current method can be applied for newborn screening for other inherited metabolic diseases as well as for assessing treatments in clinical trials. 相似文献
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Vansteenkiste Maarten; Zhou Mingming; Lens Willy; Soenens Bart 《Canadian Metallurgical Quarterly》2005,97(3):468
Various cross-cultural researchers state that autonomy is not valued in Eastern cultures and, hence, is unlikely to predict optimal study functioning and well-being. In contrast, self-determination theory (SDT; R. M. Ryan & E. L. Deci, 2000) maintains that autonomous or volitional study motivation is universally important and should predict better learning and higher well-being, even among Chinese students. Two studies were conducted to shed light on this controversial issue. Findings from both studies indicated that autonomous study motivation positively predicts adaptive learning attitudes, academic success, and personal well-being, whereas controlled motivation was associated with higher drop-out rates, maladaptive learning attitudes, and ill-being. In addition, Study 2 revealed that parental autonomy support versus psychological control is related to more adaptive learning strategies and higher well-being and that these effects were mediated by students' relative autonomy for studying. The importance of defining autonomy as an intraindividual, phenomenological experience versus an interpersonal, culturally bounded value is discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved) 相似文献
15.
Rastegari Parvin Susilo Willy Dakhilalian Mohammad 《International Journal of Information Security》2019,18(5):619-635
International Journal of Information Security - In a designated verifier signature (DVS) scheme, the signer (Alice) creates a signature which is only verifiable by a designated verifier (Bob).... 相似文献
16.
Microbially mediated phosphine emission 总被引:1,自引:0,他引:1
There is still a lot of controversy in literature concerning the question whether a biochemical system exists enabling micro-organisms to reduce phosphate to phosphine gas. The search for so-called 'de novo synthesised' phosphine is complicated by the fact that soils, slurries, sludges, etc., which are often used as inocula, usually contain matrix bound phosphine (MBP). Matrix bound phosphine is a general term used to indicate non-gaseous reduced phosphorus compounds that are transformed into phosphine gas upon reaction with bases or acids. A study was carried out to compare the different digestion methods, used to transform matrix bound phosphine into phosphine gas. It was demonstrated that caustic and acidic digestion methods should be used to measure the matrix bound phosphine of the inoculum prior to inoculation to avoid false positive results concerning de novo synthesis. This is especially true if anthropogenically influenced inocula possibly containing minute steel or aluminium particles are used. The comparative study on different digestion methods also revealed that the fraction of phosphorus in mild steel, converted to phosphine during acid corrosion depended on the temperature. Following these preliminary studies, anaerobic growth experiments were set up using different inocula and media to study the emission of phosphine gas. Phosphine was detected in the headspace gases and its quantity and timeframe of emission depended on the medium composition, suggesting microbially mediated formation of the gas. The amount of phosphine emitted during the growth experiments never exceeded the bound phosphine present in inocula, prior to inoculation. Hence, de novo synthesis of phosphine from phosphate could not be demonstrated. Yet, microbially mediated conversion to phosphine of hitherto unknown reduced phosphorus compounds in the inoculum was evidenced. 相似文献
17.
Hahn Griebel W. Schreiber Carls J. Großfeld Steinbeck Beckel Schwaibold Lehnartz Meumann Schormüller Jesser R. Strohecker Lehnartz Diemair R. Grau Lars Erlandson Patzsch Reichard Ph. Bruno Roßmann Brüning Karl Sauer Willy Lindner Kanitz O. Windhausen Schloemer Amelung M. Steiner R. Mancke K. Höll Goreczky E. Tornow Zacher Kleinhans Bäurle 《European Food Research and Technology》1943,85(1):70-112
18.
Schloemer W. Ludorff Beckel Jesser W. Schreiber Lars Erlandsen K. Lang Schwaibold Lehnartz Amelung J. Großfeld E. Tornow Griebel Schormüller A. Bäurle Willy Lindner Diemair Schmitz Zacher A. Dresler Steinbeck Reichard R. Grau Pawletta Schoop W. Wodsak O. Windhausen G. Steinhoff Hawelka J. Ph. Bruno Roßmann Brüning Edmund Baertich Dinslage 《European Food Research and Technology》1943,85(4):348-400
19.
Kanitz Hans Hawelka Schormüller Schwaibold Griebel W. Schreiber Beckel Kortenhaus H. Wenzl Lars Erlandsen Klinc Patzsch Bäurle Diemair Adolf Hanak A. Lendle Pawletta Zacher R. Grau K. Höll Schloemer Jesser W. Wodsak Lindner Masling Haevecker Steinbeck J. Großfeld E. Tornow K. Müller W. Ludorff W. Hämmerle Willy Lindner Brüning O. Windhausen E. Dinslage C. Pyriki Reichard R. Strohecker Spitta Kluge Krzywanek R. Nitsche 《European Food Research and Technology》1942,84(3):230-288
20.
Karl Sauer Diemair E. Dinslage Kanitz Schwaibold Willy Lindner Haevecker Lehnartz E. Becker W. Schreiber Werner Hofmann Eduard Hofmann Steinbeck H. Süllmann Griebel Rudolf Abderhalden Lars Erlandsen Heyns O. v. Soden C. Steinhoff J. Großfeld Frey-Wyssling W. Sutthoff Bäurle Willy Lindner Ed. Rentz Bandow Gerards K. N. v. Kaulla O. v. Soden Maria Cicconi Pawletta Baars R. Grau Lerche Beckel O. Windhausen Felicitas Rolleri Jesser Karl Boresch R. W. Seuffert K. Müller Schloemer K. Höll Johannes Wolf Brüning Patzsch W. Ludorff 《European Food Research and Technology》1941,81(3):223-288